Ten-year experience with nephrogenic systemic fibrosis: Case-control analysis of risk factors

Objectives: To analyze all cases of nephrogenic systemic fibrosis (NSF) at our institution and to compare them with controls. Methods: After the institutional review board approval, 13 biopsy-proven NSF cases were identified. Ten cases had complete records and were compared in a case-control format with 10 age- and sex-matched, dialysis-dependent controls. Analyzed risk factors included single and cumulative gadolinium dose, medication and transplant history, and serum electrolytes at the time of gadolinium exposure. Results: There were 1.9% of dialysis-dependent, gadolinium-exposed patients who developed NSF. There was no difference in gadolinium dose, transplant history, or serum electrolytes. Seven of 10 cases and 3 of 10 controls were treated with erythropoietin (P = 0.13). At the time of NSF diagnosis, 7 of 10 cases were on immunosuppressive therapy. Two of 7 cases developed NSF only after immunosuppressive therapy wasinitiated. Two of 10 controls were on immunosuppressive therapy (P = 0.06). Conclusions: All cases of NSF occurred in dialysis-dependent, gadolinium-exposed patients. Associations between immunosuppressive and erythropoietin therapies and NSF need further investigation.